Childhood brain stem glioma is a disease in which benign
(noncancer) or malignant (cancer) cells form in the tissues of the brain
stem.

The brain stem is the
part of the brain connected to the spinal cord. It is in the lowest
part of the brain, just above the back of the neck. The brain stem is the part
of the brain that controls breathing, heart rate, and the nerves and muscles used
in seeing, hearing, walking, talking, and eating. Most childhood brain stem gliomas are pontine gliomas, which form in a part of the brain stem called the pons.

EnlargeAnatomy of the brain. The supratentorial area (the upper part of the brain) contains the cerebrum, lateral ventricle and third ventricle (with cerebrospinal fluid shown in blue), choroid plexus, hypothalamus, pineal gland, pituitary gland, and optic nerve. The posterior fossa/infratentorial area (the lower back part of the brain) contains the cerebellum, tectum, fourth ventricle, and brain stem (pons and medulla). The tentorium separates the supratentorium from the infratentorium (right panel). The skull and meninges protect the brain and spinal cord (left panel).

This summary refers to the treatment of primary brain tumors (tumors that begin in the
brain). Treatment for metastatic brain tumors, which are tumors formed by cancer cells that
begin in other parts of the body and spread to the brain, is not discussed in
this summary.

Brain tumors can occur in both children and adults; however,
treatment for children may be different than treatment for adults. See the following PDQ treatment summaries for more information:

Brain tumors may be benign (not cancer) or malignant (cancer).

Benign brain tumors grow and press on nearby areas of the brain. They rarely spread into other tissues. Malignant brain tumors are likely to grow quickly and spread into other brain tissue. When a tumor grows into or presses on an area of the brain, it may stop that part of the brain from working the way it should. Both benign and malignant brain tumors can cause signs and symptoms and need treatment.

There are two types of brain stem gliomas in children.

DIPG is a high-grade tumor that is fast-growing and spreads all through the brain stem. It is hard to treat and has a poor prognosis (chance of recovery). Children younger than 3 years diagnosed with DIPG may have a better prognosis than children who are 3 years and older.

A focal glioma is slow-growing and is in one area of the brain stem. It is easier to treat than DIPG and has a better prognosis.

The cause of most childhood brain tumors is unknown.

Anything that increases your risk of getting a disease is called a risk factor. Having a risk factor does not mean that you will get cancer; not having risk factors doesn’t mean that you will not get cancer. Talk with your child's doctor if you think your child may be at risk. Possible risk factors for brain stem glioma include:

Some tumors do not cause signs or symptoms. Signs and symptoms may be caused by childhood brain stem gliomas or by other conditions. Check with your child's doctor if your child has any of the following:

Tests that examine the brain are used to detect (find) childhood brain stem glioma.

The following tests and procedures may be used:

Physical exam and history: An exam of the body to check general signs of health, including checking for signs of disease, such as lumps or anything else that seems unusual. A history of the patient’s health habits and past illnesses and treatments will also be taken.

Neurological exam: A series of questions and tests to check the brain, spinal cord, and nerve function. The exam checks a person’s mental status, coordination, and ability to walk normally, and how well the muscles, senses, and reflexes work. This may also be called a neuro exam or a neurologic exam.

MRI (magnetic
resonance imaging) with gadolinium: A procedure that uses a magnet, radio waves, and a computer to make a series of detailed pictures of areas inside the brain. A substance called gadolinium is injected into a vein. The gadolinium collects around the cancer cells so they show up brighter in the picture. This procedure is also called nuclear magnetic resonance imaging (NMRI).

A biopsy may be done to diagnose certain types of brain stem glioma.

If the MRI scan looks like the tumor is a DIPG, a biopsy is usually not done and the tumor is not removed.

If the MRI scan looks like a focal brain stem glioma, a biopsy may be done. A part of the skull is removed and a needle is used to remove a sample of the brain tissue. Sometimes, the needle is guided by a computer. A pathologist views the tissue under a microscope to look for cancer cells. If cancer cells are found, the doctor will remove as much tumor as safely possible during the same surgery.

EnlargeCraniotomy: An opening is made in the skull and a piece of the skull is removed to show part of the brain.

The following test may be done on the tissue that was removed:

Immunohistochemistry: A test that uses antibodies to check for certain antigens in a sample of tissue. The antibody is usually linked to a radioactive substance or a dye that causes the tissue to light up under a microscope. This type of test may be used to tell the difference between brain stem glioma and other brain tumors.

Treatment Option Overview

Key Points

There are different types of treatment for children with brain
stem glioma.

Children with brain stem glioma should have their treatment
planned by a team of health care providers who are experts in treating childhood brain
tumors.

Childhood brain stem gliomas may cause signs or symptoms that begin before the cancer is diagnosed and continue for months or years.

Some cancer treatments cause side effects months or years after treatment has ended.

Six types of standard treatment are used:

Surgery

Radiation therapy

Chemotherapy

Cerebrospinal fluid diversion

Observation

Targeted therapy

New types of treatment are being tested in clinical trials.

Patients may want to think about taking part in a clinical trial.

Patients can enter clinical trials before, during, or after starting their cancer treatment.

Follow-up tests may be needed.

There are different types of treatment for children with brain
stem glioma.

Different types of treatment are available for children with brain stem glioma. Some treatments
are standard (the currently used treatment), and some are being tested in clinical trials. A treatment
clinical trial is a research study meant to help improve current treatments or
obtain information on new treatments for patients with cancer. When clinical trials show that
a new treatment is better than the standard
treatment, the new treatment may become the standard treatment.

Because cancer in children is rare, taking part in a clinical trial
should be considered. Some clinical trials are open only to patients who have not started treatment.

Children with brain stem glioma should have their treatment
planned by a team of health care providers who are experts in treating childhood brain
tumors.

Childhood brain stem gliomas may cause signs or symptoms that begin before the cancer is diagnosed and continue for months or years.

Childhood brain stem gliomas may cause signs or symptoms that continue for months or years. Signs or symptoms caused by the tumor may begin before diagnosis. Signs or symptoms caused by treatment may begin during or right after treatment.

Some cancer treatments cause side effects months or years after treatment has ended.

These are called late effects. Late effects may include the following:

Some late effects may be treated or controlled. It is important to talk with your child's doctors about the effects cancer treatment can have on your child. (See the PDQ summary on Late Effects of Treatment for Childhood Cancer for more information).

Six types of standard treatment are used:

Surgery

Surgery may be used to diagnose and treat childhood brain stem glioma as discussed in the General Information section of this summary.

Radiation therapy

Radiation therapy is
a cancer treatment that uses high-energy x-rays or
other types of radiation to kill cancer cells or keep them from growing. There are two types of
radiation therapy:

The way the radiation therapy is given depends on the type of the cancer being treated.
External radiation therapy is used to treat DIPG. External and/or internal radiation therapy may be used to treat focal brain stem gliomas.

Several months after radiation therapy to the brain, imaging tests may show changes to the brain tissue. These changes may be caused by the radiation therapy or may mean the tumor is growing. It is important to be sure the tumor is growing before any more treatment is given.

Chemotherapy

Chemotherapy is a cancer treatment that uses drugs to stop the growth of
cancer cells, either by killing the cells or by stopping them from
dividing. When chemotherapy is taken by mouth or injected into a vein or muscle, the drugs enter the bloodstream and can reach cancer
cells throughout the body (systemic chemotherapy). When chemotherapy is
placed directly in the cerebrospinal fluid, an organ, or a body cavity such as the abdomen, the drugs mainly affect cancer cells in those areas (regional chemotherapy). The way the chemotherapy is given depends on the type of the cancer being treated.

Because radiation therapy to the brain can affect growth and brain development
in young children, clinical trials are studying ways of using chemotherapy to
delay or reduce the need for radiation therapy.

Cerebrospinal fluid diversion

Cerebrospinal fluid diversion is a method used to drainfluid that has built up in the brain. A shunt (long, thin tube) is placed in a ventricle (fluid-filled space) of the brain and threaded under the skin to another part of the body, usually the abdomen. The shunt carries extra fluid away from the brain so it may be absorbed elsewhere in the body.EnlargeCerebrospinal fluid (CSF) diversion. Extra CSF is removed from a ventricle in the brain through a shunt (tube) and is emptied into the abdomen. A valve controls the flow of CSF.

Observation

Targeted therapy

Targeted therapy is a type of treatment that uses drugs or other substances to identify and attack specific cancer cells without harming normal cells.

Some focal brain stem gliomas that cannot be removed by surgery may be treated with BRAF kinase inhibitor therapy. BRAF kinase inhibitors block the BRAF protein. BRAF proteins help control cell growth and may be mutated (changed) in some types of brain stem glioma. Blocking mutated BRAF kinase proteins may help keep cancer cells from growing.

New types of treatment are being tested in clinical trials.

Patients may want to think about taking part in a clinical trial.

For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment.

Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment.

Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward.

Patients can enter clinical trials before, during, or after starting their cancer treatment.

Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment.

Clinical trials are taking place in many parts of the country. See the Treatment Options section that follows for links to current treatment clinical trials. These have been retrieved from NCI's listing of clinical trials.

Follow-up tests may be needed.

Some of the tests that were done to diagnose the cancer or to find out the stage of the cancer may be repeated. Some tests will be repeated in order to see how well the treatment is working. Decisions about whether to continue, change, or stop treatment may be based on the results of these tests.

Some of the tests will continue to be done from time to time after treatment has ended. The results of these tests can show if your child's condition has changed or if the cancer has recurred (come back). These tests are sometimes called follow-up tests or check-ups.

If the results of imaging tests done after treatment show a mass in the brain, a biopsy may be done to find out if it is made up of dead tumor cells or if new cancer cells are growing. In children who are expected to live a long time, regular MRIs may be done to see if the cancer has come back.

Treatment of brain stem glioma in children with neurofibromatosis
type 1 may be observation. The tumors are slow-growing in these children
and may not need specific treatment for years.

Check the list of NCI-supported cancer clinical trials that are now accepting patients with untreated childhood brain stem glioma. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your child's doctor about clinical trials that may be right for your child. General information about clinical trials is available from the NCI website.

Progressive or Recurrent Childhood Brain Stem Glioma

When cancer does not get better with treatment or comes back, palliative care is an important part of the child's treatment plan. It includes physical, psychological, social, and spiritual support for the child and family. The goal of palliative care is to help control symptoms and give the child the best quality of life possible. Parents may not be sure about whether to continue treatment or what kind of treatment is best for their child. The healthcare team can give parents information to help them make these decisions.

Check the list of NCI-supported cancer clinical trials that are now accepting patients with recurrent childhood brain stem glioma. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your child's doctor about clinical trials that may be right for your child. General information about clinical trials is available from the NCI website.

About This PDQ Summary

About PDQ

Physician Data Query (PDQ) is the National Cancer Institute's (NCI's) comprehensive cancer information database. The PDQ database contains summaries of the latest published information on cancer prevention, detection, genetics, treatment, supportive care, and complementary and alternative medicine. Most summaries come in two versions. The health professional versions have detailed information written in technical language. The patient versions are written in easy-to-understand, nontechnical language. Both versions have cancer information that is accurate and up to date and most versions are also available in Spanish.

PDQ is a service of the NCI. The NCI is part of the National Institutes of Health (NIH). NIH is the federal government’s center of biomedical research. The PDQ summaries are based on an independent review of the medical literature. They are not policy statements of the NCI or the NIH.

Purpose of This Summary

This PDQ cancer information summary has current information about the treatment of childhood brain stem glioma. It is meant to inform and help patients, families, and caregivers. It does not give formal guidelines or recommendations for making decisions about health care.

Reviewers and Updates

Editorial Boards write the PDQ cancer information summaries and keep them up to date. These Boards are made up of experts in cancer treatment and other specialties related to cancer. The summaries are reviewed regularly and changes are made when there is new information. The date on each summary ("Date Last Modified") is the date of the most recent change.

Clinical Trial Information

A clinical trial is a study to answer a scientific question, such as whether one treatment is better than another. Trials are based on past studies and what has been learned in the laboratory. Each trial answers certain scientific questions in order to find new and better ways to help cancer patients. During treatment clinical trials, information is collected about the effects of a new treatment and how well it works. If a clinical trial shows that a new treatment is better than one currently being used, the new treatment may become "standard." Patients may want to think about taking part in a clinical trial. Some clinical trials are open only to patients who have not started treatment.

Clinical trials are listed in PDQ and can be found online at NCI's website. Many cancer doctors who take part in clinical trials are also listed in PDQ. For more information, call the Cancer Information Service 1-800-4-CANCER (1-800-422-6237).

Permission to Use This Summary

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Updated: June
9, 2017

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